JPGN reports最新文献

{"title":"Nudix hydrolase 15 deficiency and azathioprine toxicity in a liver transplant recipient: A case report.","authors":"Jenny Q Nguyen, Natalie Rodriguez, Kimberly A Chapman, Matthew A Deardorff, Keith Z Hazleton","doi":"10.1002/jpr3.70243","DOIUrl":"https://doi.org/10.1002/jpr3.70243","url":null,"abstract":"<p><p>Thiopurines, such as azathioprine (AZA), are among the immunosuppressants used to prevent allograft rejection. Thiopurines are metabolized by thiopurine S-methyltransferase (TPMT) and nudix hydrolase 15 (NUDT15) enzymes, and their functions are highly influenced by genetic variation. We present a 6-year-old female with recurrent acute cellular rejection and steroid-induced glaucoma due to high-dose corticosteroid therapy. Consequently, AZA was incorporated into her calcineurin-inhibitor-based regimen to reduce steroid dose. Within 33 days of initiating AZA, alopecia and pancytopenia developed despite confirmed TPMT enzyme activity with normal phenotype and low 6-Thioguanine and 6-Methylmercaptopurine levels in the blood. Pharmacogenomic analysis revealed an <i>NUDT15</i> *3/*3 genotype, indicating two \"no function\" alleles, classified as NUDT15 poor metabolizer at high risk of myelosuppression with standard thiopurine doses. Liver donor pharmacogenomic testing was not performed. This case highlights the consideration of pharmacogenomic testing for recipient and donor prior to thiopurine use in liver-transplant recipients.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13662887/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149198649","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Resolution of proximal tubular dysfunction after liver transplantation in ATP8B1 deficiency: A case report.","authors":"Angela Marino, Nathalie Godefroid, Catherine De Magnée, Xavier Stephenne","doi":"10.1002/jpr3.70258","DOIUrl":"https://doi.org/10.1002/jpr3.70258","url":null,"abstract":"<p><p>Progressive familial intrahepatic cholestasis type 1 (PFIC1), caused by ATP8B1 deficiency, is a multisystem disorder with extrahepatic manifestations; however, renal involvement has rarely been described. We report a child with genetically confirmed PFIC1 who underwent living donor liver transplantation (LT) for progressive liver disease. Before transplantation, serum creatinine was normal, but a Fanconi-like proximal tubular dysfunction was characterized by elevated urinary β2-microglobulin, proteinuria, phosphaturia, hypercalciuria, and metabolic acidosis requiring bicarbonate supplementation. Following transplantation, these abnormalities rapidly resolved, supporting the hypothesis of reversible cholestasis-associated tubular injury. This case identifies proximal tubular dysfunction as an underrecognized, potentially reversible manifestation of severe, prolonged cholestasis in PFIC1 and supports systematic evaluation of tubular markers before transplantation.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13659308/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149187122","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Assessing the current landscape of North American pediatric nutrition support teams: A survey study.","authors":"Rachel E Herdes, Lily Xia, Atu Agawu, Arun Ajmera, Mary Abigail S Garcia, Rahiya Rehman, Kanika Puri, Michael Carver, Danielle Wendel, Vijay Mehta, Bridget Dowd Kiernan, Tanyaporn K Kaenkumchorn, Rachel Knight, Nicole Martin, Alexandra Carey, Jennifer Panganiban, Deb Duro","doi":"10.1002/jpr3.70256","DOIUrl":"https://doi.org/10.1002/jpr3.70256","url":null,"abstract":"<p><strong>Introduction: </strong>Nutrition support teams (NSTs) were developed to standardize nutrition evaluation practices and coordinate transdisciplinary expertise for appropriate administration of inpatient nutritional care. Minimal data are available regarding the current practices, structure, and perceived utility of pediatric NSTs in diverse hospital systems.</p><p><strong>Methods: </strong>This was a cross-sectional survey study disseminated electronically to three electronic listservs associated with the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN).</p><p><strong>Results: </strong>Forty-seven unique responses were obtained. Sixty-two percent reported a current NST at their institution. Of those with an NST, 97% reported reviewing parenteral nutrition (PN) orders, 90% recommended nutritional assessment modalities, 86% determined PN appropriateness, and 72% reviewed enteral nutrition orders. More than 50% of respondents strongly agreed that an NST improved patient safety, improved patient nutritional status, improved quality of care, decreased rates of malnutrition, and promoted interdisciplinary care. Among those without an NST, 33% of respondents reported that they were previously unsuccessful at developing an NST at their site. Perceived barriers to NST implementation in more than 50% of respondents were lack of protected time, budget or funding issues, lack of available staff, and lack of interdisciplinary support.</p><p><strong>Discussion: </strong>This study demonstrates heterogeneity of pediatric NSTs in North America with regard to frequency of services, while showing general consistency in the scope of services provided. It highlights the overall perceived benefit of NSTs. Financial compensation continues to be a barrier to access to NSTs with a notable percentage of NSTs reportedly not billing for services.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-10-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13656294/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149187116","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Tirzepatide use in adolescents with metabolic dysfunction-associated steatotic liver disease (MASLD): A case series.","authors":"Brandon J Perumpail, Andrea Tou, Jennifer Panganiban","doi":"10.1002/jpr3.70246","DOIUrl":"https://doi.org/10.1002/jpr3.70246","url":null,"abstract":"<p><p>Pediatric obesity and metabolic dysfunction-associated steatotic liver disease (MASLD) prevalence has risen markedly over the past decade. Due to transition delays and workforce shortages, pediatric specialists often manage patients into young adulthood, and thus familiarity with adult pharmacotherapies for MASLD is essential. Currently, Semaglutide, a glucagon-like peptide-1 (GLP-1) receptor agonist, is approved for adults with metabolic dysfunction-associated steatohepatitis (MASH) and F2-F3 fibrosis; however, dual and triple agonists are being evaluated. Tirzepatide, a dual agonist that targets gastric inhibitory peptide (GIP) and the GLP-1 receptor, has demonstrated superior weight reduction to Semaglutide (-20.2% vs. -13.7%) with a more favorable gastrointestinal adverse effect profile. Adult literature has demonstrated its effectiveness in MASH resolution, and pediatric data suggest it is safe in younger populations. Currently, there are no pediatric/adolescent studies exploring the use of Tirzepatide in MASLD. We present our experience using Tirzepatide in two adolescent patients with obesity and MASLD.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-10-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13656291/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149187128","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A novel, free regional conference model to increase interest and engagement of medical trainees in pediatrics and pediatric gastroenterology research.","authors":"Mahnoor Memon, Shahzaib Khan, Christina Guillen, Thomas Wallach","doi":"10.1002/jpr3.70252","DOIUrl":"https://doi.org/10.1002/jpr3.70252","url":null,"abstract":"<p><p>Declining interest in pediatrics, contraction of the pediatric gastroenterology fellowship applicant pool, and the long-standing erosion of the physician-scientist workforce threaten the future of pediatric academic medicine. Cost and logistical barriers to traditional academic conferences disproportionately affect trainees from underrepresented or resource-limited backgrounds and may compound these pipeline pressures. We developed Eats, Streets, and Beets (ESB), a free, trainee-centered regional pediatric nutrition conference held in Brooklyn, NY, combining faculty-led symposia, guaranteed podium presentations for every accepted abstract, and a community block party. A post-conference survey (<i>n</i> = 12; response rate 63.2%) demonstrated high overall satisfaction (mean 8.25/10) and self-reported increases in interest in research, pediatrics, and pediatric gastroenterology and nutrition. Trainee-only programming, podium-only presentations, and community engagement were most strongly associated with increased interest. This low-cost, replicable model may help reinvigorate trainee engagement across the pediatric and pediatric gastroenterology pipelines.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-10-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13656292/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149186903","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Acneiform eruption associated with Janus kinase inhibitors in pediatric inflammatory bowel disease: Case series and clinical management guidance.","authors":"Tina H Tran, Daniel Park, Regina-Celeste Ahmad, Malika Goel, Sofia Verstraete, Sabina Ali","doi":"10.1002/jpr3.70255","DOIUrl":"https://doi.org/10.1002/jpr3.70255","url":null,"abstract":"<p><p>Janus kinase inhibitors (JAKi) are increasingly used for refractory inflammatory bowel disease (IBD) in pediatric patients but are associated with significant dermatologic adverse effects. Acneiform eruptions have been reported in up to 30% of adults, with a greater incidence suspected in adolescents. We performed a retrospective review of 66 pediatric IBD patients treated with JAKi at UCSF Benioff Children's Hospitals (2022-2025), including 41 on upadacitinib and 25 on tofacitinib. Eight patients (12.1%) developed new-onset or worsening moderate to severe acne requiring dermatology referral for management; rates were higher with upadacitinib (6/41, 14.6%) than tofacitinib (2/25, 8.0%). Acne onset was early (median 3.5 weeks) and predominantly inflammatory. Four patients required JAKi discontinuation, and two developed acne fulminans requiring hospitalization. Management often required systemic therapy and multidisciplinary care. These findings highlight the need for anticipatory counseling, early recognition, and prompt dermatologic involvement to reduce morbidity and support continuation of effective IBD therapy.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-10-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13656293/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149187113","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A comparison of the upright seated versus left lateral decubitus position during pediatric sedation-free transnasal endoscopy.","authors":"Catherine Nguyen, Molly Mackensen, Sophie Branen, Katherine Vaidy, Rose Lee","doi":"10.1002/jpr3.70248","DOIUrl":"10.1002/jpr3.70248","url":null,"abstract":"<p><strong>Objectives: </strong>Although sedation-free transnasal endoscopy (TNE) is conventionally performed in an upright seated position in pediatric patients, emerging data suggest that the left lateral decubitus (LLD) position may improve both endoscopic visualization and patient comfort and safety. This study compared TNE outcomes and tolerance in pediatric patients using the upright versus LLD position.</p><p><strong>Methods: </strong>A retrospective review was conducted on 110 pediatric patients who underwent sedation-free TNE from September 2024 to November 2025. Three cases were excluded for scope failure during the procedure. Data including patient demographics, procedure time, TNEase (patient tolerance) score, and adverse events were collected.</p><p><strong>Results: </strong>Of the 107 cases, 71 were performed upright, 31 LLD, and 5 converted from upright to LLD due to syncope. Four cases of emesis and two cases of minor epistaxis occurred during upright TNE, while no major adverse events occurred during LLD TNE. There were no significant differences between upright and LLD TNE in patient age (upright 13.8 ± 2.8 vs. LLD 12.5 ± 3.3 years), mean TNEase score (1.1 vs. 1.4), or procedure times. For TNE-esophagoscopy, mean procedure times were 5.1 (<i>n</i> = 55) and 5.5 min (<i>n</i> = 27) in the upright versus LLD position, respectively. Times were 6.1 (<i>n</i> = 12) versus 6.8 min (<i>n</i> = 4) for TNE-esophagogastroscopy, and 17.7 (<i>n</i> = 4) versus 17.4 min (<i>n</i> = 5) for TNE-esophagogastroduodenoscopy.</p><p><strong>Conclusions: </strong>The LLD position is a safe and effective alternative to upright TNE and is better tolerated in patients with orthostatic intolerance.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13640396/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149121791","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Group ingestion of high-powered magnets in children-A clinical and public health challenge.","authors":"Anat Sigal, Inna Novak","doi":"10.1002/jpr3.70244","DOIUrl":"https://doi.org/10.1002/jpr3.70244","url":null,"abstract":"<p><p>High-powered magnets are a preventable cause of serious gastrointestinal injury in children, with ingestion often resulting in hospitalization and occasionally surgery. We report six classmates, aged 5-6 years, who ingested high-powered magnets during a single school day after mistaking them for candy. All presented to our emergency department within 12 h of ingestion. Three underwent endoscopy and three were managed with serial imaging; all magnets were retrieved or passed spontaneously, with no obstruction, perforation, or surgery. The near-simultaneous presentations strained endoscopy resources and required coordinated triage by magnet number, configuration, and symptoms. The U.S. Consumer Product Safety Commission defines these magnets for users ≥14 years, yet all patients were under 7, underscoring age-based labeling limitations. To our knowledge, this is the first reported cluster of simultaneous classroom magnet ingestion, highlighting persistent exposure despite regulation and the need for standardized triage protocols and national surveillance.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13625144/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149121802","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Ultrasound findings associated with neonatal acute liver failure secondary to gestational alloimmune liver disease: A case series.","authors":"Naseem Ravanbakhsh, Ashley M Upton, Chun-Sing Huang, Nhu Thao N Galván, John Goss, Sanjiv Harpavat, Jose A Hernandez","doi":"10.1002/jpr3.70241","DOIUrl":"10.1002/jpr3.70241","url":null,"abstract":"<p><strong>Objectives: </strong>Gestational alloimmune liver disease is the leading cause of neonatal acute liver failure, hypothesized to emerge from maternal exposure to an antigen that is expressed on fetal hepatocytes, resulting in maternal-fetal alloimmune attack and activation of the complement cascade. Despite maximal medical therapy, some require liver transplantation for survival. Prompt recognition is vital. Ultrasound is the first imaging modality utilized in acute liver failure. Results often include nonspecific findings. There is a need for more sonographic findings to facilitate timely diagnosis. We present a case series of neonates who presented with acute liver failure secondary to gestational alloimmune liver disease at Texas Children's Hospital from 2018 to 2025.</p><p><strong>Methods: </strong>This study was approved by the Baylor College of Medicine International Review Board H40231. Medical records, imaging studies, and biopsy results from 2018 to 2025 were analyzed retrospectively. A General Electric LOGIQ E10 ultrasound machine was used.</p><p><strong>Results: </strong>All four patients with gestational alloimmune liver disease shared four findings on ultrasound: a widely patent ductus venosus, small right portal veins, reversal of flow in the right portal vein, and innumerable liver lesions.</p><p><strong>Conclusion: </strong>Our sonographic findings can be used to prompt clinical suspicion for gestational alloimmune liver disease and expedite treatment.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13602002/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149043263","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The ImproveCareNow Registry: Prediction of different remission rates in pediatric ulcerative colitis.","authors":"Jonathan Van Hecke, Gigi Veereman, Koen Huysentruyt, Lucas Wauters, Jeremy Adler, Shehzad Saeed, Elisabeth De Greef","doi":"10.1002/jpr3.70234","DOIUrl":"10.1002/jpr3.70234","url":null,"abstract":"<p><strong>Objectives: </strong>Identifying predictors of clinical remission in pediatric ulcerative colitis (UC) will impact the therapeutic approach. We aimed to predict time to first (TFR), corticosteroid-free (TCSFR, ≥3 months corticosteroid withdrawal), and sustained remission (TSR, ≥6 months).</p><p><strong>Methods: </strong>This retrospective study analyzed de-identified prospectively collected data from 2383 patients enrolled in ImproveCareNow (2007-2023). We included UC patients under 18 years at diagnosis. Remission was defined as a Pediatric UC Activity Index (PUCAI; <10 points). The Statistical Package for the Social Sciences version 29 with multiple imputation for missing data was used for statistical analysis. Cox regression identified predictors at diagnosis/enrollment (T0) or first visit (T1) on the outcomes (<i>n</i> = 1785). This study received ethics committee approval (23135-ICN_UC).</p><p><strong>Results: </strong>Mean TFR was 177.7 days, predicted by disease extent (<i>p</i> > 0.05) at T0 and history of severe UC (hazard ratio [HR] = 1.142, <i>p</i> = 0.05) before inclusion. In patients not reaching remission at T1 (subgroup; <i>n</i> = 964), TFR was predicted by PUCAI (confounder; moderate HR = 0.867 or severe disease HR = 0.697, <i>p</i> < 0.1) at T1 and history of severe UC (HR = 1.292, <i>p</i> = 0.007) before T1 (mean 25 days SEM 0.7). Mean TCSFR was 569.6 days, predicted by PUCAI (only mild HR = 0.728 and moderate HR = 0.559, <i>p</i> < 0.001) and use of corticosteroids (HR = 0.668, <i>p</i> < 0.001) at T1 (mean 32 days SEM 0.9). Mean TSR was 631.0 days, predicted by PUCAI (only mild HR = 0.721 and moderate HR = 0.541, <i>p</i> < 0.001) and the use of oral aminosalicylates (HR = 0.793, <i>p</i> = 0.003) at T1 (mean 30 days SEM 0.7).</p><p><strong>Conclusions: </strong>Several clinical parameters in early disease course predict remission with increasing PUCAI as useful negative predictor.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-09-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13574566/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148979057","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}